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HCCS Antibody

  • 中文名稱:
    HCCS兔多克隆抗體
  • 貨號:
    CSB-PA010165GA01HU
  • 規(guī)格:
    ¥3,900
  • 其他:

產(chǎn)品詳情

  • Uniprot No.:
  • 基因名:
    HCCS
  • 別名:
    CCHL antibody; CCHL_HUMAN antibody; cytochrome c heme-lyase antibody; Cytochrome c-type heme lyase antibody; DKFZp779I1858 antibody; EC 4.4.1.17 antibody; Hccs antibody; Holocytochrome c synthase (cytochrome c heme lyase) antibody; Holocytochrome c synthase antibody; Holocytochrome c type synthase antibody; Holocytochrome c-type synthase antibody; MCOPS7 antibody; OTTHUMP00000022903 antibody; OTTHUMP00000022904 antibody; OTTHUMP00000022905 antibody; OTTMUSP00000021173 antibody; OTTMUSP00000021174 antibody; RGD1563855 antibody; RP23-37L2.1 antibody
  • 宿主:
    Rabbit
  • 反應(yīng)種屬:
    Human,Mouse,Rat
  • 免疫原:
    Human HCCS
  • 免疫原種屬:
    Homo sapiens (Human)
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen Affinity purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產(chǎn)品提供形式:
    Liquid
  • 應(yīng)用范圍:
    ELISA,WB,IHC
  • Protocols:
  • 儲存條件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期:
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評價

靶點詳情

  • 功能:
    Lyase that catalyzes the covalent linking of the heme group to the cytochrome C apoprotein to produce the mature functional cytochrome.
  • 基因功能參考文獻:
    1. Data suggest that although HCCS mediates heme attachment to N-terminal cysteine in heme-attachment site (CXXXH) of cytochrome C variants, up to 50% of cytochrome C produced is modified in an oxygen-dependent manner, resulting in a mixed population of cytochrome c. [HCCS = holocytochrome c synthase] PMID: 28617588
    2. Bacterial cyt c biogenesis pathways (Systems I and II) appear to recognize simply the CXXCH motif, not requiring alpha helix-1. Results here explain mechanistically how HCCS (System III) requires an extended region adjacent to CXXCH for maturation. PMID: 27387500
    3. cysteines and histidine of the heme attachment site, Cys-XX-Cys-His play a key role in mitochondrial holocytochrome c synthase PMID: 25170082
    4. These data indicate that heme contacts mediated by residues within these domains modulate the dynamics of heme binding and contribute to the stability of the HCCS-heme-cytochrome c steady state ternary complex. PMID: 25054239
    5. Identification of a deletion in two patients, including HCCS, leads to the diagnosis of microphthalmia with linear skin defects syndrome PMID: 23401659
    6. Spectroscopic analyses of HCCS alone and complexes of HCCS with site-directed variants of cytochrome c revealed the fundamental steps of heme attachment and maturation. PMID: 23150584
    7. Through the study of genetically engineered mice, the loss of HCCS is demonstrated to cause the male lethality of microphthalmia with linear skin defects (MLS) syndrome. PMID: 12444108
    8. mutant proteins (R217C and Delta 197-268) were unable to complement a Saccharomyces cerevisiae mutant deficient for the HCCS orthologue Cyc3p, in contrast to wild-type HCCS PMID: 17033964
    9. Missense mutation p.E159K of HCCS, leading to loss-of-function of encoded holocytochrome c-type synthase, in female with microphthalmia of both eyes and bilateral sclerocornea may suggest HCCS as candidate for severe ocular manifestations. PMID: 17893649

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  • 相關(guān)疾病:
    Linear skin defects with multiple congenital anomalies 1 (LSDMCA1)
  • 亞細胞定位:
    Mitochondrion inner membrane. Membrane; Lipid-anchor.
  • 蛋白家族:
    Cytochrome c-type heme lyase family
  • 數(shù)據(jù)庫鏈接:

    HGNC: 4837

    OMIM: 300056

    KEGG: hsa:3052

    STRING: 9606.ENSP00000326579

    UniGene: Hs.211571



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